Chronic interstitial lung disease associated with systemic lupus erythematosus : A multicentric study of 89 cases
© 2024 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology..
BACKGROUND AND OBJECTIVE: Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE-ILD from the OrphaLung and French SLE networks during 2005-2020 aimed to describe the characteristics of patients with SLE-ILD and analyse factors associated with prognosis.
METHODS: We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE.
RESULTS: Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression.
CONCLUSION: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2024 |
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| Erschienen: |
2024 |
| Enthalten in: |
Zur Gesamtaufnahme - year:2024 |
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| Enthalten in: |
Respirology (Carlton, Vic.) - (2024) vom: 17. März |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Deneuville, Lou [VerfasserIn] |
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| Links: |
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| Themen: |
Connective-tissue disease |
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| Anmerkungen: |
Date Revised 18.03.2024 published: Print-Electronic Citation Status Publisher |
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| doi: |
10.1111/resp.14703 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| PPN (Katalog-ID): |
NLM369845188 |
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| 100 | 1 | |a Deneuville, Lou |e verfasserin |4 aut | |
| 245 | 1 | 0 | |a Chronic interstitial lung disease associated with systemic lupus erythematosus |b A multicentric study of 89 cases |
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| 500 | |a Date Revised 18.03.2024 | ||
| 500 | |a published: Print-Electronic | ||
| 500 | |a Citation Status Publisher | ||
| 520 | |a © 2024 The Authors. Respirology published by John Wiley & Sons Australia, Ltd on behalf of Asian Pacific Society of Respirology. | ||
| 520 | |a BACKGROUND AND OBJECTIVE: Chronic interstitial lung disease (ILD) occurs rarely with systemic lupus erythematosus (SLE) as compared with other connective tissue diseases. This multicentric retrospective study of patients with SLE-ILD from the OrphaLung and French SLE networks during 2005-2020 aimed to describe the characteristics of patients with SLE-ILD and analyse factors associated with prognosis | ||
| 520 | |a METHODS: We analysed data for 89 patients with SLE-ILD (82 women, 92.1%) (median age at SLE diagnosis: 35 years [interquartile range 27-47]). All patients met the 2019 EULAR/ACR criteria for the diagnosis of SLE | ||
| 520 | |a RESULTS: Forty two (47.2%) patients were positive for anti-ribonuclear protein antibodies and 45 (50.6%) for anti SSA/Ro antibodies. A total of 58 (65.2%) patients had another connective tissue disease: Sjögren's syndrome (n = 33, 37.1%), systemic sclerosis (n = 14, 15.7%), inflammatory myopathy (n = 6, 6.7%), or rheumatoid arthritis (n = 6, 6.7%). ILD was diagnosed along with SLE in 25 (28.1%) patients and at a median of 6 (0-14) years after the SLE diagnosis. The most frequent CT pattern was suggestive of non-specific interstitial pneumonia (n = 41, 46.0%) with or without superimposed organizing pneumonia. After a median follow-up of 86.5 [39.5-161.2] months, 18 (20.2%) patients had died and 6 (6.7%) underwent lung transplantation. The median 5-year and 10-year transplantation-free survival were 96% (92-100) and 87% (78-97). In total, 44 (49.4%) patients showed ILD progression. Cutaneous manifestations and Raynaud's phenomenon were associated with better survival. Only forced vital capacity was significantly associated with survival and ILD progression | ||
| 520 | |a CONCLUSION: ILD is a rare manifestation of SLE with good overall prognosis but with possible risk of ILD progression. Patients with SLE-ILD frequently have another connective tissue disease | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a connective-tissue disease | |
| 650 | 4 | |a disease progression | |
| 650 | 4 | |a interstitial lung disease | |
| 650 | 4 | |a lupus | |
| 650 | 4 | |a non-specific interstitial pneumonia | |
| 650 | 4 | |a pulmonary fibrosis | |
| 650 | 4 | |a survival | |
| 700 | 1 | |a Mageau, Arthur |e verfasserin |4 aut | |
| 700 | 1 | |a Debray, Marie Pierre |e verfasserin |4 aut | |
| 700 | 1 | |a Sacre, Karim |e verfasserin |4 aut | |
| 700 | 1 | |a Costedoat-Chalumeau, Nathalie |e verfasserin |4 aut | |
| 700 | 1 | |a Hachulla, Eric |e verfasserin |4 aut | |
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| 700 | 1 | |a Crestani, Bruno |e verfasserin |4 aut | |
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| 700 | 1 | |a Audia, Sylvain |e investigator |4 oth | |
| 700 | 1 | |a Nunes, Hilario |e investigator |4 oth | |
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